Dunns EXTRA stuff Flashcards
supplementary carbohydrate metabolism
What are glycosaminoglycans?
GAGS
- long unbranched polysaccharides
- repeating disaccharide unit of hexosamine or hexose sugar and uronic acid or hexose sugar
- e.g. Heparin
What can the hexosamines or the hexose sugar be in the GAG?
Hexosamine:
N-acetylglucosamine or N-acetylgalactosamine
Hexose Sugar:
Galactose
What can the uronic acid or the hexose sugar be in the GAG?
Uronic Acid:
Glucuronic acid or iduronic acid
Hexose Sugar:
Galactose
When are the disaccharides in GAGS sulfated?
Most cases
- exception is hyaluronic acid
What are glycosamioglycans disorders?
- lysosomal storage disorders
- inherited diseases called MPS diseases
- defects in lysosomal enzymes associated with metabolism/ breakdowns of GAGs leading to an accumulation of GAGs in cells
Name and describe a MPS disease.
Sanfilippo disease - rare - primarily effects heparan sulfate - causes mental deterioration Effects cornea, heart, lungs, brain and mental functions
Where are GAGs prevalent?
In proteoglycans
What is the structure of proteoglycans?
- protein with one of multiple (>100) GAGs covalently attached
- up to 95% of proteoglycan
- trisaccharide links GAGs to the protein
- large molecules
What are trisaccharides in GAGs composed of?
2 galactose residues and a xylose residue
What are the properties of GAGs?
- highly negatively charged
- highly hydrophilic
What can GAGs do in the body?
- concentrate cations such as sodium
- can form porous hydrated gels that are active in the extracellular matrix providing mechanical support, infused with chains
- can form long chains (withstand compression forces and act as lubricants around joints)
- chains are inflexible
What are glycoproteins?
- oligosaccharide chains covalently linked to proteins
Name an example of a glycoprotein.
Erythropoietin (EPO) is a glycoprotein hormone
- controls rbc production (haemopoiesis)
What are glycoproteins usually composed of?
No GAGs
Usually, glucose, galactose manose etc
- not typically sulfated
How does the ratio of glycoproteins compare to that or proteoglycans?
Protein:sugar ratio is higher, more proteins than hexose sugars in glycoproteins than in proteoglycans
How do we acquire fatty acids?
Through diet
Where can we synthesise fatty acids?
Liver and adipose fat
What are the 4 fatty acid synthesis steps?
- condensation of acetyl-ACP and malonyl ACP
- reduction
- dehydration
- reduction to form butyryl-ACP
- further processes add malonyl-acp to synthesise a c16 or c18 fatty acid